Malignant peripheral nerve sheath tumor
Alerts and Notices
Synopsis

Neurofibromas may transform into MPNSTs. This is of specific concern in patients with neurofibromatosis, who may have a high number of peripheral nerve tumors.
MPNSTs have a poor prognosis generally, with a worse prognosis in people with large tumors or neurofibromatosis. The primary treatment is surgical resection.
Codes
ICD10CM:C47.9 – Malignant neoplasm of peripheral nerves and autonomic nervous system, unspecified
SNOMEDCT:
404037002 – Malignant peripheral nerve sheath tumor
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
- Neurofibroma
- Schwannoma
- Perineurioma
- Ganglioneuroma
- Metastatic cancer
- Neurolymphomatosis
- Local hematoma with nerve compression
- Neuroma
- Trauma (see traumatic neuroma)
- Vasculitis
- Nerve infarction
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
References
Subscription Required
Last Reviewed:04/04/2019
Last Updated:04/22/2019
Last Updated:04/22/2019